Hoogendijk J E, Bijlsma J W J, van Engelen B G M, Lindeman E, van Royen-Kerkhof A, de Rie M A, de Visser M, Jennekens F G I
Universitair Medisch Centrum Utrecht, Utrecht.
Ned Tijdschr Geneeskd. 2005 Sep 17;149(38):2104-11.
This guideline presents recommendations for the diagnosis and treatment of dermatomyositis, polymyositis and sporadic inclusion body myositis (sIBM) according to the best available evidence. Characteristic skin abnormalities can be sufficient for the diagnosis of dermatomyositis. In case of doubt, a skin biopsy is advisable. A muscle biopsy is indicated when other examinations are inconclusive and the musculature is involved. The working group considers screening for cancer to be required in adults with dermatomyositis and presents recommendations for the way that this should be done. At least one-third of all patients with polymyositis has, or will develop, an associated inflammatory connective tissue disease. If a patient with a connective tissue disease develops symmetrical, proximal muscle weakness in the course of weeks or months, this may be assumed to be due to polymyositis. In the absence ofpre-existing connective tissue disease, demonstration of a mononuclear cell infiltrate in muscle tissue is a prerequisite for the diagnosis ofpolymyositis. The histopathology of muscle tissue is used as the gold standard for the diagnosis of sIBM. The practice guideline presents criteria for the concept 'activity' of myositis. Disease activity serves as a guideline for the treatment of polymyositis and dermatomyositis. The treatment of choice for dermatomyositis and polymyositis is high-dose prednisone. Physical activity does not have a negative effect on the course of these diseases. The long-term prognosis ofdermatomyositis and polymyositis is not well known. The clinical course of sIBM is slowly progressive.
本指南依据现有最佳证据，给出了皮肌炎、多发性肌炎和散发性包涵体肌炎（sIBM）的诊断与治疗建议。特征性皮肤异常足以诊断皮肌炎。如有疑问，建议进行皮肤活检。当其他检查结果不明确且肌肉组织受累时，需进行肌肉活检。工作组认为，成年皮肌炎患者需要进行癌症筛查，并给出了筛查方式的建议。所有多发性肌炎患者中，至少三分之一患有或将会患上相关的炎症性结缔组织病。如果患有结缔组织病的患者在数周或数月内出现对称性近端肌无力，则可认为是由多发性肌炎所致。在无既往结缔组织病的情况下，肌肉组织中出现单核细胞浸润是诊断多发性肌炎的必要条件。肌肉组织的组织病理学检查是诊断sIBM的金标准。本实践指南给出了肌炎“活动度”概念的标准。疾病活动度是多发性肌炎和皮肌炎治疗的指导依据。皮肌炎和多发性肌炎的首选治疗方法是大剂量泼尼松。体育活动对这些疾病的病程没有负面影响。皮肌炎和多发性肌炎的长期预后尚不清楚。sIBM的临床病程呈缓慢进展。
